Unusual infection causes dementia

DR. PAUL DONOHUE GOOD HEALTH

April 3, 2009|DR. PAUL DONOHUE GOOD HEALTH

Dear Dr. Donohue: My dad died of confirmed sporadic CJD, Creutzfeldt-Jakob disease. He had a very short and terrible illness. Although this is a very rare disease, will you explain it to make more doctors and families aware of it? - D.G.

Dear D.G.: Only about 300 cases of Creutzfeldt-Jakob are diagnosed yearly in the United States. It is an awful illness, caused by a most unusual infectious agent, a prion. Prions are remarkable life forms. They are proteins, and they lack nucleic acid, something all other living things have. Most Creutzfeldt-Jakob disease is sporadic - happening in a random fashion to people without a known source of transmission.

It's a dementia, an illness where there is a deterioration of mental function. With CJD, the dementia is rapid, occurring in a matter of months. The infected person loses memory, reasoning, judgment and even the basics of a thinking life. Myoclonus is another hallmark of the illness. It's sudden, profound jerks of the arms or legs or other muscles. A loud noise or any stimulus can cause this exaggerated reaction.

Dear Dr. Donohue: I am a 41-year-old woman, very petite, 5 feet 1 inch tall, and weigh 95 to 105 pounds. I am being bullied about this by my doctors. Have they supersized the height-weight tables to accommodate our increasingly obese population? I am a lightweight but have no health problems. Your thoughts are welcome. - L.N.

Dear L.N.: Your body mass index is 18.9, which puts you in the normal weight category but at its lower end. If you feel healthy and eat a balanced diet, bully those doctors back.